A LOOK INTO MYASTHENIA GRAVIS Edited by Joseph A. Pruitt A Look into Myasthenia Gravis Edited by Joseph A. Pruitt Published by InTech Janeza Trdine 9, 51000 Rijeka, Croatia Copyright © 2011 InTech All chapters are Open Access distributed under the Creative Commons Attribution 3.0 license, which allows users to download, copy and build upon published articles even for commercial purposes, as long as the author and publisher are properly credited, which ensures maximum dissemination and a wider impact of our publications. After this work has been published by InTech, authors have the right to republish it, in whole or part, in any publication of which they are the author, and to make other personal use of the work. Any republication, referencing or personal use of the work must explicitly identify the original source. 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Publishing Process Manager Oliver Kurelic Technical Editor Teodora Smiljanic Cover Designer InTech Design Team First published January, 2012 Printed in Croatia A free online edition of this book is available at www.intechopen.com Additional hard copies can be obtained from orders@intechweb.org A Look into Myasthenia Gravis, Edited by Joseph A. Pruitt p. cm. ISBN 978-953-307-821-2 free online editions of InTech Books and Journals can be found at www.intechopen.com Contents Preface VII Part 1 Clinical Presentations 1 Chapter 1 Myasthenia Gravis with Anti-MuSK Antibodies: Clinical Features and Histopathological Changes 3 Corrado Angelini, Sara Martignago, Michela Biscigli and Elisa Albertini Chapter 2 Ocular Manifestations of Myasthenia Gravis 15 Joseph A. Pruitt and Pauline Ilsen Part 2 Treatments 21 Chapter 3 Myasthenia Gravis – Current Treatment Standards and Emerging Drugs 23 Kamil Musilek, Marketa Komloova, Ondrej Holas, Anna Horova, Jana Zdarova-Karasova and Kamil Kuca Chapter 4 Immunomodulatory Treatments for Myasthenia Gravis: Plasma Exchange, Intravenous Immunoglobulins and Semiselective Immunoadsorption 43 Fulvio Baggi and Carlo Antozzi Chapter 5 Respiratory Care for Myasthenic Crisis 55 Ping-Hung Kuo and Pi-Chuan Fan Part 3 Surgical Considerations 75 Chapter 6 Unilaterally Extended Thoracoscopic Thymectomy: The Right Side or the Left Side Approach 77 Victor Tomulescu Chapter 7 Robotic Thymectomy 95 Victor Tomulescu Preface This publication is the result of the joining of forces, so to speak, of a diverse group of professionals in an effort to depict the many manifestations of myasthenia gravis. Although, in the grand scheme of things, only a few manifestations are covered in this text, the far-reaching effect of this disease is not lost. From the surgical treatment of the disease's complications to the common findings of a routine eye examination, this text explores this disease's impact in a variety of outlets within healthcare. We hope you find this text interesting, but most importantly informative. Enjoy. Joseph A. Pruitt Minneapolis Veterans Affairs Medical Center, Minneapolis, Minnesota, University of Missouri-Saint Louis College of Optometry, Saint Louis, Missouri USA Part 1 Clinical Presentations [...]... Development of Generalized Myasthenia Gravis in Patinets With Ocular Myasthenia Gravis Arch Neurol 2003; 60(10):1491-1492 Hilton-Jones D, Palace J The management of myasthenia gravis Practical Neurology 2005;5:18-27 18 A Look into Myasthenia Gravis http://www.ninds.nih.gov/disorders /myasthenia_ gravis /myasthenia_ gravis. htm Golnik K How to Diagnose and Treat Myasthenia Gravis Review of Ophthalmology 2002;9(10):219...1 Myasthenia Gravis with Anti-MuSK Antibodies: Clinical Features and Histopathological Changes 1Department Corrado Angelini1,2, Sara Martignago1, Michela Biscigli1 and Elisa Albertini1 of Neurosciences, University of Padova, Padova 2IRCCS San Camillo; Venezia Italy 1 Introduction Myasthenia gravis (MG) is a neuromuscular autoimmune disorder caused by binding of autoantibodies to molecules... by age and gender and MG was first thought to be a condition of the young female and old male Epidemiological data show that women are most frequently affected than men; age at onset in MG patients with MuSK-Ab occurs predominantly before 40 years, at a younger age than MG cases with AChR-Ab [9] Women Myasthenia Gravis with Anti-MuSK Antibodies: Clinical Features and Histopathological Changes 5 are... MG, and has to be analised as a peculiar muscle pathology Treatment and diagnosis, as well as prognosis and surgical approach is different in MG with MuSK-Ab The clinician should be alerted of this different bfeatures and have a different approach to this tipe of myasthenia gravis 7 Anti-MuSK patients in clinical practice In our Neuromuscular Centre Database we collected 279 MG patients: 171 female and... neuroimmunological diseases J Neurol 2006 Sep; 253 Suppl 5:V2-8 [11] Phillips LH, 2nd The epidemiology of myasthenia gravis Ann N Y Acad Sci 2003 Sep; 998:407-12 [12] Thanvi BR, Lo TC Update on myasthenia gravis Postgrad Med J 2004 Dec; 80 (950):690-700 [13] Myasthenia Gravis Foundation of America Myasthenia gravis: a manual for the health care provider 2008 [cited; Available from: http://www myasthenia. org/docs/... ultrastructure in myasthenia gravis with anti-MuSK and anti-AChR antibodies J Neurol 2010 Nov 19; 258:746–752 [29] Appel SH, Anwyl R, McAdams MW, Elias S Accelerated degradation of acetylcholine receptor from cultured rat myotubes with myasthenia gravis sera and globulins Proc Natl Acad Sci USA 1977; 74: 2124–30 [30] Rostedt Punga A, Ahlqvist K, Bartoccioni E, Scuderi F, Marino M, Suomalainen A, Kalimo... in abnormal neuromuscular transmission and muscle weakness Approximately 80% of patients have detectable serum antibodies against AChR that reduce AChR number and impair their function at the neuromuscular endplate About 15-30% of MG patients do not have detectable AChRAb and are defined as seronegative These patients may have purely ocular symptoms but a small proportion has generalized weakness About... matrix and fragmented cristae [28] Mitochondria are aggregated both in the subsarcolemmal and intermyofibrillar areas (Figure 3), adjacent to normal mitochondria Myasthenia Gravis with Anti-MuSK Antibodies: Clinical Features and Histopathological Changes 9 Fig 1 Light microscopy Acid ATP-ase stain Fig 2 COX stain shows 2 COX-negative shows type I fibre grouping in one AChR-Ab fibers (star) in one AChR-Ab... dysphagia with an associated high risk of aspiration and those who are unable to ambulate without assistance Although slower-acting than plama exchange, the response is similar and offers advantages when therapeutic plasmaphesis is not available or when vascular access is problematic Significant improvement is seen in patients whose therapy consist of an initial dose of 400 mg/kg/d for 5 days and followed... Sewry C Myasthenic syndromes In: Muscle biopsy A practical approach 3rd ed Saunders Elsevier, London 2007: 512-515 14 A Look into Myasthenia Gravis [27] Martignago S, Fanin M, Albertini E, Pegoraro E, Angelini C Muscle histopathology in myasthenia gravis with antibodies against MuSK and AChR Neuropathol Appl Neurobiol 2009; 35:103–110 [28] Cenacchi G, Papa V, Fanin M, Pegoraro E, Angelini C Comparison . Part 2 Treatments 21 Chapter 3 Myasthenia Gravis – Current Treatment Standards and Emerging Drugs 23 Kamil Musilek, Marketa Komloova, Ondrej Holas, Anna Horova, Jana Zdarova-Karasova and. Features and Histopathological Changes 3 Corrado Angelini, Sara Martignago, Michela Biscigli and Elisa Albertini Chapter 2 Ocular Manifestations of Myasthenia Gravis 15 Joseph A. Pruitt and Pauline. b1745272 M II aXXXX+II a6 49431 F * ACH. anticholineterases; STER. Steroid; IM. Immunosoppressive drugs; PE. Plasma exchange ^ AS asintomatic A Look into Myasthenia Gravis 8 AChR-Ab patients.